J Ped Surg Case Reports 3 (2015) 389e391

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Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

A rare variant case of pure esophageal atresia with an atretic

segment
Kazunori Masahata a, *, Toshimichi Hasegawa a, Seika Kuroda a, Nobumasa Takahashi b,
Ikuko Nojima b, Noriko Kimura b, Keiji Tsuchimoto b, Akio Kubota c
a
Department of Pediatric Surgery, National Hospital Organization Fukuyama Medical Center, Okinogamicho 4-4-17, Fukuyama City, Hiroshima 720-
8520, Japan
b
Department of Neonatology, National Hospital Organization Fukuyama Medical Center, Okinogamicho 4-4-17, Fukuyama City, Hiroshima 720-8520,
Japan
c
Second Department of Surgery, Wakayama Medical University, 811-1, Kimiidera, Wakayama-shi, Wakayama 641-8510, Japan

a r t i c l e i n f o a b s t r a c t

Article history: Pure esophageal atresia is typically characterized by a long gap between the upper and lower pouches,
Received 11 June 2015 with a gasless abdomen and no fistula. The association of pure esophageal atresia with an atretic
Received in revised form segment is extremely rare. We report a rare variant case of pure esophageal atresia in which the two
3 August 2015
blind esophageal pouches were joined by an atretic segment. Excision of the atretic segment and primary
Accepted 4 August 2015
anastomosis were performed successfully.
Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Key words:
Esophageal atresia
Kluth’s classification
Atretic segment

Pure esophageal atresia (PEA) is typically characterized by a long
gap between the upper and lower pouches, with a gasless abdomen
and no fistula [1]. PEA is found in about 5e7% of all cases of
esophageal atresia (EA) [2]. A variety of anatomical subtypes have
been reported in PEA [3]. The association of PEA with an atretic
segment is extremely rare, and only a few cases have been reported
[2,4,5]. We report a rare case of PEA corresponding to subtype II3 EA
in Kluth’s atlas [3], with two blind esophageal pouches connected
by an atretic segment.
1. Case report
A 2.8-kg male baby was born at 36 weeks of gestation by
Cesarean section. Prenatal ultrasonography showed poly-
hydramnios and absence of a fetal gastric fluid bubble, suggesting
Author contribution: Conception and design: K.M., T.H. Data collection: K.M., S.K.,
T.H., N.T., I.N., N.K., K.T. Data interpretation: K.M., S.K., T.H. Drafting the manuscript:
K.M., T.H. Contribution to the final manuscript and important intellectual content:
K.M., T.H., A.K.
* Corresponding author. Tel.: þ81 84 922 0001; fax: þ81 84 931 3969.
E-mail address: masahata_kazunori@fukuyama-hosp.go.jp (K. Masahata).
EA of Gross type A. Apgar score was 8 at both 1 and 5 min. He
presented with drooling of saliva and a flat abdomen, but no
associated anomalies. In the neonatal intensive care unit, a
nasogastric tube was not able to be passed into the stomach, and
radiological examination showed a gasless abdomen. On the basis
of these findings, EA of Gross type A was diagnosed. Temporary
gastrostomy was performed and bronchoscopy revealed no evi-
dence of tracheoesophageal fistula (TEF) on the first day of life.
Contrast study via the gastrostomy suggested that the distance
between the esophageal segments was at least two vertebral
bodies (Fig. 1). The decision was made at the time to delay
attempted primary anastomosis until after elongation of the up-
per pouch by manual bougienage. By 3 months old, both ends of
the esophageal segments overlapped on radiography (Fig. 2).
Mediastinal exploration was performed through a right extrap-
leural thoracotomy. The upper esophageal pouch appeared
markedly enlarged. The blind ends of the esophagus were
confirmed to be connected by an atretic segment about 20 mm
long and 2 mm thick (Fig. 3). A feeding tube inserted by the
anesthetist was not able to be passed through the beginning of
this segment. The atretic segment was excised, the blind ends
opened and primary end-to-end esophageal anastomosis was
performed. On histological examination, the atretic segment

2213-5766/Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
http://dx.doi.org/10.1016/j.epsc.2015.08.003
390 K. Masahata et al. / J Ped Surg Case Reports 3 (2015) 389e391
Fig. 1. Preoperative contrast study via gastrostomy demonstrates a gap of at least two
vertebral bodies between the upper and lower esophageal pouches.
Fig. 2. Chest radiography demonstrates both ends of the esophagus overlapping at 3
months old.
Fig. 3. Intraoperative photograph shows the upper esophageal pouch (E) and an atretic
segment (arrow) that connects both esophageal pouches.
consisted of smooth muscle layers with a fibrous core and no
lumen. These findings were consistent with subtype II3 in Kluth’s
atlas of EA (Fig. 4) [3]. Although the postoperative course was
uneventful, a contrast study demonstrated mild anastomotic
stenosis and gastroesophageal reflux on postoperative day 10. The
patient was maintained on antireflux medication after the surgery
to decrease the risk of gastroesophageal reflux disease. Oral
feeding progressed gradually from postoperative day 14. The pa-
tient was discharged on postoperative day 40, and as of 5 months
old appears to be developing well.
2. Discussion
EA is characterized by interruption to the continuity of the
esophagus with or without fistula to the trachea. The incidence of
EA is generally 1 in approximately 2500e5000 live births [6]. The
commonly used classifications are described by Vogt [7] and Gross
[8]. A variety of anatomical subtypes of EA not covered by these
Fig. 4. Diagrammatic representation of the variant anatomy of type II3 EA according to
the Kluth atlas (a) and histologic examination of an atretic segment (b) showing
smooth muscle layers with a fibrous core without any lumen. Our case was classified as
this type of PEA. Hematoxylin and eosin staining. UP, upper esophageal pouch; At,
atretic segment; LP, lower esophageal pouch.
 K. Masahata et al. / J Ped Surg Case Reports 3 (2015) 389e391 391

Table 1
Summary of patients with variant subtype II3 EA.

Case Reported Author Sex Age at Preoperative Treatment Length Histological findings Postoperative
year definitive elongation of of atretic band course
surgery atretic
band
1 1984 Wada et al. F 5 mo Mechanical elongation Delayed primary anastomosis NA Smooth muscles without a lumen NA
2 1999 Katsuno et al. F 5 mo NA Delayed primary anastomosis 3.7 cm Smooth muscles without a lumen NA
3 1999 Katsuno et al. M 3 mo NA Delayed primary anastomosis 2.5 cm Smooth muscles without a lumen NA
4 2007 Sanal et al. [2] M 0 d No treatment Early primary anastomosis 1 cm Smooth muscles, nonfunctional Good
lumina and blood vessels
5 2009 Dutta et al. [4] M 2d No treatment Early primary anastomosis 2.4 cm Striated muscles groups without Good
a lumen
6 2010 Torikai et al. NA 5 mo Mechanical elongation Delayed primary anastomosis NA NA Good
7 2011 Yokoi et al. NA 2 mo No treatment Delayed primary anastomosis NA NA NA
8 2012 Nishi et al. [5] M 3 mo No treatment Delayed primary anastomosis 2 cm Smooth muscles and tracheal Good
cartilage without a lumen
9 2015 Present report M 3 mo Mechanical elongation Delayed primary anastomosis 2 cm Smooth muscles and nerve Good
fibers without a lumen

M, male; F, female; mo, months; d, days; NA, not available.

classifications have been reported. Kluth published a complete
listing of all described variations of EA in 1976, including 10
separate classes and additional subclasses [3]. He placed the forms
of PEA as type II. In this group of esophageal anomalies, he clas-
sified 5 subtypes. In our case, the upper and lower esophageal
pouches were connected by an atretic segment without a lumen
(Fig. 3). According to Kluth’s classification, this type of PEA would
be classified as subtype II3, in which the midportion of the
esophagus is atretic without a TEF. This type of PEA is extremely
rare. We identified 8 other cases of Kluth type II3 described in the
literature, with the findings summarized in Table 1 [2,4,5]. Ages at
definitive surgery ranged from 0 days to 5 months (mean, 2.8
months) and the length of the atretic segment ranged from 1.0 cm
to 3.7 cm (mean, 2.2 cm). Six cases underwent delayed primary
repair following initial gastrostomy, whereas two cases underwent
curative surgery without gastrostomy shortly after birth. The most
accepted treatment strategy for PEA is delayed primary repair
following initial gastrostomy for feeding purposes, because of the
long gap between pouches. In three cases, the upper esophageal
pouch was able to be sufficiently elongated by manual bougienage
preoperatively, so primary esophageal anastomosis was performed
after excising the atretic segment. In all cases, esophageal anas-
tomosis was successfully achieved, and the postoperative course
was uneventful.
Various theories have been put forward, but the pathogenesis of
PEA with an atretic segment are unclear. Mechanical hypotheses
involving factors such as intraembryonic pressure, vascular occlu-
sion, failure of recanalizaion, and disproportionate growth of the
lateral folds do not appear sufficient to explain the whole spectrum
of this anomaly [9]. Kluth and Habenicht [10] suggested that the
pathogeneses of PEA and EA with TEF differ from those of the
combined form of EA with fistula between the distal pouch and
trachea. They demonstrated that PEA is probably due to partial
necrosis of the esophagus caused by local microcirculatory disor-
ders. According to the classification of intestinal atresia, this type of
PEA with an atretic segment is similar to type II atresia of the small
intestine, which involves two atretic blind ends connected by a
band of fibrous cord [4,5]. The pathogenesis of this variant could be
explained on the basis of the prenatal vascular accident theory
proposed by Louw and Barnard for intestinal atresia [11]. In 1955,
they demonstrated in experiments that interruption of blood flow
caused intestinal atresia to various degrees in dog fetuses.
Tsujimoto et al. [12] verified in an experiment that ischemic in-
testine causing a vascular obstruction became necrotic and was
resorbed, with the wall comprising fibrous tissues and smooth
muscle. As a result, we think that such a lesion becomes an atretic
segment with the viable ends connected by a fibrous cord. However,
only one case with tracheal cartilage in the atretic segment occur-
ring in association with PEA has been described [5]. Such findings
suggest that the present type of PEA may be caused by disturbance
of the blood supply at an early stage in embryonic development and
foregut malformation.
In conclusion, we have presented a rare variant of PEA with an
atretic segment, corresponding to subtype II3 EA in Kluth’s atlas. In
such cases, esophageal anastomosis may be performed successfully
and lead to good prognosis.
Disclosure of funding
None.
Conflict of interest
The authors declare that they have no conflicts of interest.
Sources of support
None.
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